title: Hereditary ectodermal dysplasia: A retrospective study.
abstract: BACKGROUND
  Ectodermal dysplasia (ED) is a group of rare, inherited disorders characterized by sparse hair, missing teeth and inability to sweat.
  
  
  OBJECTIVE
  To review and analyze cases of ED with an emphasis on clinical manifestations and parent's marriage history.
  
  
  METHODS
  The present retrospective study was conducted by assessing the clinical records of nineteen cases of ED, available in the archives of the department; for age, gender, family history of consanguineous marriage and clinical manifestations.
  
  
  RESULTS
  It was observed that ED was more prevalent in males, with a ratio of 1.7:1. The hypohydrotic type was more common (78.95%) than hydrotic type (21.05%). The marriage history of parents revealed that 66.67% had consanguineous marriage and had 68.42% offspring's affected with ED; whereas 33.33% had history of non-consanguineous marriage and had 31.58% offspring's affected with ED. The clinical manifestations observed were- dry skin(94.74%); scaly skin(42.11%); sparse hair on scalp, eyebrows and eyelashes(100%); frontal bossing(63.18%); saddle nose (57.89%); hypertelorism (47.37%); nail abnormality(52.63%); normal sweat glands(21.05%); abnormal sweat glands(78.95%); hypoplastic maxilla(52.63%); protuberant lips (57.89%); palmo-plantar keratosis(21.05%); wrinkled & hyper pigmented facial skin(84.21%); partial anodontia(94.74%); conical shaped teeth(84.21%); high arched palate(68.42%); thin alveolar bone(100.00%); taurodontism(21.05%) and cleft lip & cleft palate(05.26%). The number of teeth present in all the cases ranged from 0 to 19.
  
  
  CONCLUSIONS
  ED patients suffer from social problems and poor psychological and physiological development as a result of unacceptable esthetics and abnormal function of orofacial structures. Oral rehabilitation thus becomes mandatory, although it is often difficult; particularly in pediatric patients.
authors.affiliation: Department of Oral Medicine and Radiology, K. M. Shah Dental College and Hospital, Sumandeep Vidyapeeth, Piparia, Vadodara, Gujarat, India.
authors.forename: Chandramani B
authors.initials: CB
authors.lastname: More
authors.forename: Khusbhu
authors.initials: K
authors.lastname: Bhavsar
authors.forename: Jigar
authors.initials: J
authors.lastname: Joshi
authors.forename: Saurabh N
authors.initials: SN
authors.lastname: Varma
authors.forename: Mansi
authors.initials: M
authors.lastname: Tailor
keywords: Anodontia; consanguineous marriage; ectodermal dysplasia; hydrotic; hypohydrotic; hypotrichosis; taurodontism